No abstract

Background: In Pompe disease, resistance exercise could be an effective treatment to delay motor function impairment, however, the acute effects of this exercise modality are unclear.Methods: In a prospective cohort study, we compared responses to a single bout of resistance exercise by serum markers of muscle damage and quantitative muscle magnetic resonance imaging (MRI) in patients (n=12) and age-and gender-matched healthy controls (n=12). Participants performed 50 maximal effort concentric knee flexions on a dynamometer.Results: Twenty-four hours after exercise, levels of serum creatine kinase, lactate dehydrogenase and myoglobin increased in controls. In contrast, only myoglobin level increased in patients. All elevated serum markers declined by 48 hours after exercise in both groups. Mild soreness developed at 24 hours, which disappeared at 48 hours in both groups. In controls, MRI R2* relaxation rate reduced immediately and 24 hours after exercise, indicating increased water content and muscle perfusion. In patients, exercise had no effect on R2* values. The resistance exercise did not induce acute strength deficit in patients, rather, patients increased their strength by 24 hours. When serum marker changes were normalized to the magnitude of knee flexor tension developed during exercise, lactate dehydrogenase response was greater in patients.Conclusions: Late-onset Pompe disease did not exacerbate exercise-induced muscle damage, however, lactate dehydrogenase may be monitored to screen high responders during high intensity resistance exercise interventions.